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  • Open Access


    Strategy of treating secundum atrial septal defect not referred to percutaneous closure

    Fei Zhang1, Yifeng Yang2, Qin Wu2, Wancun Jin2, Haisong Bu2, Sijie Wu2, Tianli Zhao2, Shijun Hu2

    Congenital Heart Disease, Vol.14, No.3, pp. 324-330, 2019, DOI:10.1111/chd.12753

    Abstract Objective: To evaluate safety and effectiveness of intraoperative device closure for secundum atrial septal defect (ASD) not referred to percutaneous closure.
    Design and Patients: From April 2010 to December 2018, 231 secundum ASD children (≤14 years) directly recommended to surgical repair were enrolled in this study. These patients were divided into two groups according to the parents’ choice based on surgeons’ recommendation. Follow-up evaluations were adopted at 2 weeks, 3 months, 6 months, and 12 months after the procedure and yearly thereafter. In Group A, 127 patients underwent an initial attempt at device closure. In Group B,… More >

  • Open Access


    Safety of contraceptive use among women with congenital heart disease: A systematic review

    Ginnie Abarbanell1, Naomi K. Tepper2, Sherry L. Farr3

    Congenital Heart Disease, Vol.14, No.3, pp. 331-340, 2019, DOI:10.1111/chd.12752

    Abstract Objective: Women with congenital heart disease (CHD) are at increased risk of preg‐ nancy complications and need information on safe, effective contraceptive methods to avoid unintended pregnancy. This systematic review examines evidence regarding safety of contraceptive use among women with CHD.
    Methods: The PubMed database was searched for any peer‐reviewed articles pub‐ lished through April 2018 that included safety outcomes associated with reversible contraceptive methods among women with CHD.
    Results: Five articles met inclusion criteria: three studies comparing contraceptive users to nonusers and two noncomparative studies. Sample sizes ranged from 65 to 505 women with CHD. Two studies… More >

  • Open Access


    Body mass index in adults with congenital heart disease

    Mahmoud Zaqout1,2, Kristof Vandekerckhove1, Nathalie Michels2, Laurent Demulier3, Thierry Bove4, Katrien François4, Julie De Backer3, Stefaan De Henauw2, Daniel De Wolf1

    Congenital Heart Disease, Vol.14, No.3, pp. 479-486, 2019, DOI:10.1111/chd.12751

    Abstract Objective: To investigate the status of body mass index (BMI) in adult people with congenital heart disease (ACHD).
    Methods: Five hundred thirty‐nine adults with CHD (53.8% men) were seen in the outpatient clinic from 2013 to 2015 and compared to a reference population (n = 1737). The severity of CHD was categorized as mild, moderate, and severe ac‐ cording to standard guidelines. Patients were categorized based on BMI as under‐ weight (<18.5), overweight (25‐30), or obese (>30). Echocardiography and magnetic resonance imaging were used to measure ventricular function while exercise capac‐ ity was estimated via cardiopulmonary exercise… More >

  • Open Access


    Accuracy of risk prediction scores in pregnant women with congenital heart disease

    Yuli Y. Kim1,2, Leah A. Goldberg2, Katherine Awh2, Tanmay Bhamare1,2, David Drajpuch2, Adi Hirshberg3, Sara L. Partington1,2, Rachel Rogers4, Emily Ruckdeschel1,2, Lynda Tobin1, Morgan Venuti2, Lisa D. Levine3

    Congenital Heart Disease, Vol.14, No.3, pp. 470-478, 2019, DOI:10.1111/chd.12750

    Abstract Objective: To assess performance of risk stratification schemes in predicting adverse cardiac outcomes in pregnant women with congenital heart disease (CHD) and to compare these schemes to clinical factors alone.
    Design: Single‐center retrospective study.
    Setting: Tertiary care academic hospital.
    Patients: Women ≥18 years with International Classification of Diseases, Ninth Revision, Clinical Modification codes indicating CHD who delivered between 1998 and 2014. CARPREG I and ZAHARA risk scores and modified World Health Organization (WHO) criteria were applied to each woman.
    Outcome Measures: The primary outcome was defined by ≥1 of the following: arrhyth‐ mia, heart failure/pulmonary edema, transient ischemic attack, stroke,… More >

  • Open Access


    A better approach for left ventricular training in transposition of the great arteries and intact interventricular septum: Bidirectional cavopulmonary anastomosis and pulmonary artery banding

    Mehmet Salih Bilal1, Arda Özyüksel1,2, Mustafa Kemal Avşar1, Şener Demiroluk3, Osman Küçükosmanoğlu4, Yalım Yalçın5

    Congenital Heart Disease, Vol.14, No.3, pp. 464-469, 2019, DOI:10.1111/chd.12749

    Abstract Objective: Management of the patients with transposition of the great arteries and intact ventricular septum may be challenging beyond the newborn period. Herein, we would like to present our alternative strategy for training the left ventricle in these patients.
    Methods: Six patients with transposition of the great arteries and intact ventricular septum were evaluated in our clinic. Two of them were palliated with Glenn procedure and pulmonary banding as a definitive treatment strategy at other centers. Four patients were operated on and a bidirectional cavopulmonary anastomosis in combination with pulmonary artery banding was performed (stage‐1: palliation and… More >

  • Open Access


    Opportunities for training to advance the care for adults with congenital heart disease with advanced circulatory failure

    Christopher R. Broda

    Congenital Heart Disease, Vol.14, No.3, pp. 487-490, 2019, DOI:10.1111/chd.12748

    Abstract Heart failure is an emerging issue with important implications in adult patients with congenital heart disease. Practitioners with expertise in both adult congenital heart disease and heart failure are needed to manage this growing and often complex pop‐ ulation. In the United States, the optimal training pathway to enable practitioners to best care for these patients is ill‐defined. This article explores possibilities and issues that interested trainees may encounter during their training experience. More >

  • Open Access


    Education as important predictor for successful employment in adults with congenital heart disease worldwide

    Maayke A. Sluman1,2, Silke Apers3,4, Judith K. Sluiter1,*, Karen Nieuwenhuijsen1, Philip Moons4,5, Koen Luyckx6,7, Adrienne H. Kovacs8,9, Corina Thomet10, Werner Budts11, Junko Enomoto12, Hsiao‐Ling Yang13, Jamie L. Jackson14, Paul Khairy15, Stephen C. Cook16, Raghavan Subramanyan17, Luis Alday18, Katrine Eriksen19, Mikael Dellborg20,21, Malin Berghammer5,22, Eva Mattsson23, Andrew S. Mackie24, Samuel Menahem25, Maryanne Caruana26, Kathy Gosney27, Alexandra Soufi28, Susan M. Fernandes29, Kamila S. White30, Edward Callus31, Shelby Kutty32, Berto J. Bouma33, Barbara J.M. Mulder33

    Congenital Heart Disease, Vol.14, No.3, pp. 362-371, 2019, DOI:10.1111/chd.12747

    Abstract Background: Conflicting results have been reported regarding employment status and work ability in adults with congenital heart disease (CHD). Since this is an impor‐ tant determinant for quality of life, we assessed this in a large international adult CHD cohort.
    Methods: Data from 4028 adults with CHD (53% women) from 15 different countries were collected by a uniform survey in the cross‐sectional APPROACH International Study. Predictors for employment and work limitations were studied using general linear mixed models.
    Results: Median age was 32 years (IQR 25‐42) and 94% of patients had at least a high school degree. Overall… More >

  • Open Access


    Creatinine‐based estimation of glomerular filtration rate in patients with a Fontan circulation

    Thomas G. Wilson1,2, Yves d’Udekem1,2,3, David S. Winlaw4,5, Rachael L. Cordina5,6, Julian Ayer4,5, Thomas L. Gentles7, Robert G. Weintraub1,2,8, Leeanne E. Grigg9, Michael Cheung1,8, Timothy M. Cain10, Padma Rao10, Charlotte Verrall4, Karin Du Plessis1, Kathryn Rice7, Ajay J. Iyengar1,3

    Congenital Heart Disease, Vol.14, No.3, pp. 454-463, 2019, DOI:10.1111/chd.12746

    Abstract Background: Patients with a Fontan circulation are at risk of renal dysfunction. We analyzed cross‐sectional data in pediatric and adult Fontan patients in order to assess the accuracy of commonly used serum creatinine‐based methods in estimating glo‐ merular filtration rate (GFR). Methods: A total of 124 Fontan patients (58 children, 66 adults) were enrolled across three study centers. Measurement of GFR (mGFR) using in vivo 99mTc‐DTPA clear‐ ance was performed. Various serum creatinine‐based equations were used to calcu‐ late estimated GFR (eGFR). Results: Mean mGFR was 108 ± 28 mL/min/1.73 m2 in children and 92 ±… More >

  • Open Access


    Electronic health record associated stress: A survey study of adult congenital heart disease specialists

    Darcy N. Marckini1, Bennett P. Samuel1, Jessica L. Parker2, Stephen C. Cook1,3

    Congenital Heart Disease, Vol.14, No.3, pp. 356-361, 2019, DOI:10.1111/chd.12745

    Abstract Background: Physician burnout has many undesirable consequences, including nega‐ tive impact on patient care delivery and physician career satisfaction. Electronic health records (EHRs) may exacerbate burnout by increasing physician workload.
    Objective: To determine burnout in adult congenital heart disease (ACHD) specialists by assessing stress associated with EHRs.
    Design: Electronic survey study of ACHD providers.
    Setting: Canada and United States.
    Participants: Three hundred eighty‐three ACHD specialists listed on the Adult Congenital Heart Association directory between February and April 2017.
    Outcome Measures: Burnout was measured using the Maslach Burnout Inventory (MBI) to understand factors contributing to work life and EHR satisfaction. Chi‐ square… More >

  • Open Access


    Circulating biomarkers of left ventricular hypertrophy in pediatric coarctation of the aorta

    Benjamin S. Frank1, Tracy T. Urban2, Karlise Lewis2, Suhong Tong3, Courtney Cassidy4, Max B. Mitchell5, Christopher S. Nichols6, Jesse A. Davidson1

    Congenital Heart Disease, Vol.14, No.3, pp. 446-453, 2019, DOI:10.1111/chd.12744

    Abstract Objective: Patients undergoing surgical repair of aortic coarctation have a 50% risk of pathologic left ventricular remodeling (increased left ventricular mass or relative wall thickness). Endothelin 1, ST2, galectin 3, norepinephrine and B‐natriuretic pep‐ tide are biomarkers that have been associated with pathologic LV change in adult populations but their predictive value following pediatric coarctation repair are not known.
    Hypothesis: Biomarker levels at coarctation repair will predict persistent left ven‐ tricular remodeling at 1‐year follow up.
    Design: Prospective, cohort study of 27 patients’ age 2 days‐12 years with coarcta‐ tion of the aorta undergoing surgical repair. Echocardiograms were… More >

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